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10岁时预测的血清肌酐倒数作为口服半胱胺治疗的肾病性胱氨酸病患儿肾功能的一项指标。

Predicted reciprocal serum creatinine at age 10 years as a measure of renal function in children with nephropathic cystinosis treated with oral cysteamine.

作者信息

Gahl W A, Schneider J A, Schulman J D, Thoene J G, Reed G F

机构信息

Human Genetics Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892.

出版信息

Pediatr Nephrol. 1990 Mar;4(2):129-35. doi: 10.1007/BF00858823.

DOI:10.1007/BF00858823
PMID:2397178
Abstract

The predicted reciprocal creatinine at age 10 years (PRC10), a parameter of renal function based upon the linear relationship between reciprocal serum creatinine and age, incorporates age, serum creatinine, and rate of renal deterioration into a single term. PRC10 measurements were employed to assess renal function in children with nephropathic cystinosis treated with oral cysteamine, a cystine-depleting agent. In 71 children receiving oral cysteamine for at least 1 year, PRC10 decreased linearly with initial serum creatinine concentration. This indicated that, although established renal damage in cystinosis was irreversible, early intervention with cysteamine therapy could favorably alter the rate of glomerular deterioration. In other analyses, mean PRC10 was shown to increase with duration of cysteamine therapy and extent of leukocyte cystine depletion. The predicted reciprocal creatinine value at a certain age can be useful in analyzing the effects of therapeutic intervention in a disease with a relatively uniform rate of renal deterioration.

摘要

10岁时预测的肌酐倒数(PRC10)是基于血清肌酐倒数与年龄之间的线性关系得出的肾功能参数,它将年龄、血清肌酐和肾功能恶化速率整合为一个单一指标。PRC10测量值被用于评估接受口服半胱胺(一种耗竭胱氨酸的药物)治疗的肾病性胱氨酸病患儿的肾功能。在71名接受口服半胱胺至少1年的儿童中,PRC10随初始血清肌酐浓度呈线性下降。这表明,尽管胱氨酸病中已确立的肾损伤是不可逆的,但早期采用半胱胺治疗可有利地改变肾小球恶化速率。在其他分析中,平均PRC10显示随半胱胺治疗持续时间和白细胞胱氨酸耗竭程度的增加而升高。在一种肾功能恶化速率相对一致的疾病中,特定年龄的预测肌酐倒数值有助于分析治疗干预的效果。

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Predicted reciprocal serum creatinine at age 10 years as a measure of renal function in children with nephropathic cystinosis treated with oral cysteamine.10岁时预测的血清肌酐倒数作为口服半胱胺治疗的肾病性胱氨酸病患儿肾功能的一项指标。
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本文引用的文献

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Effects of cysteamine therapy in nephropathic cystinosis.半胱胺治疗肾病性胱氨酸病的效果
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The treatment of cystinosis with cysteamine and phosphocysteamine in the United Kingdom and Eire.在英国和爱尔兰使用半胱胺和磷酸半胱胺治疗胱氨酸病。
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Pediatr Nephrol. 1994 Aug;8(4):466-71. doi: 10.1007/BF00856532.
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Science. 1982 Sep 24;217(4566):1263-5. doi: 10.1126/science.7112129.
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Defective cystine exodus from isolated lysosome-rich fractions of cystinotic leucocytes.胱氨酸病白细胞中富含溶酶体的分离组分的胱氨酸外排缺陷。
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Hypothyroidism in cystinosis. A clinical, endocrinologic and histologic study involving sixteen patients with cystinosis.胱氨酸病中的甲状腺功能减退症。一项涉及16例胱氨酸病患者的临床、内分泌学和组织学研究。
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Cysteamine depletes cystinotic leucocyte granular fractions of cystine by the mechanism of disulphide interchange.半胱胺通过二硫键交换机制消耗胱氨酸病白细胞颗粒部分中的胱氨酸。
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